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Abnormalities in cardiac structure and function in adults with sickle cell disease are not associated with pulmonary hypertension. J Am Soc Echocardiogr 2011 Nov;24(11):1285-90

Date

08/30/2011

Pubmed ID

21873028

Pubmed Central ID

PMC3200507

DOI

10.1016/j.echo.2011.07.009

Scopus ID

2-s2.0-80054982773 (requires institutional sign-in at Scopus site)   15 Citations

Abstract

BACKGROUND: In sickle cell disease (SCD), pulmonary hypertension (assessed by tricuspid regurgitant jet [TRJ] velocity ≥ 2.5 m/sec) is associated with increased mortality. The relationships among TRJ velocity and left ventricular (LV) and right ventricular (RV) systolic and diastolic function (i.e., relaxation and compliance) have not been well characterized in SCD.

METHODS: A prospective study was conducted in 53 ambulatory adults with SCD (mean age, 34 years; range, 21-65 years) and 33 African American controls to define the relationship between LV and RV function and TRJ velocity using echocardiography.

RESULTS: Subjects with SCD had larger left and right atrial volumes and increased LV mass compared with controls. When patients with SCD were compared with controls, LV and RV relaxation (i.e., E') were similar. Among subjects with SCD, pulmonary hypertension (TRJ ≥ 2.5 m/sec) was present in 40%. Higher TRJ velocity was correlated with larger left atrial volumes in patients with SCD. Additionally, some measures of LV (peak A, lateral and septal annular E/E' ratio) and RV (tricuspid valve E/E' ratio) compliance were correlated with TRJ velocity. No other measures of LV and RV systolic function or LV diastolic function (i.e., relaxation and compliance) were associated with TRJ velocity.

CONCLUSIONS: Ambulatory adults with SCD exhibited structural (i.e., LV and RV chamber enlargement) and functional (i.e., higher surrogate measures of LV and RV filling pressure) abnormalities compared with the control group. In subjects with SCD, few abnormalities of LV and RV structure and function were associated with TRJ velocity.

Author List

Knight-Perry JE, de Las Fuentes L, Waggoner AD, Hoffmann RG, Blinder MA, Dávila-Román VG, Field JJ

Author

Joshua J. Field MD Professor in the Medicine department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Adult
Aged
Anemia, Sickle Cell
Cardiomegaly
Comorbidity
Echocardiography, Doppler, Pulsed
Female
Humans
Hypertension, Pulmonary
Male
Middle Aged
Prospective Studies
Ventricular Dysfunction, Left
Ventricular Dysfunction, Right
Ventricular Pressure